He also discovered a syndrome in which HI is combined with hyperammonemia, an abnormal level of blood ammonia. In HI/HA, the mutation that he identified prevents an enzyme called GDH from functioning normally, leading to excessive levels of insulin, as well as childhood epilepsy, seizures and learning disabilities. Current drug treatment controls insulin levels, but not the neurological problems. The drug also has side effects, such as excessive hair growth.

Recent animal study finds compound in green tea promising

Based on a decade-long scientific collaboration, Dr. Stanley co-authored a recent study showing that a compound found in green tea controls this enzyme defect in animals, raising the possibility of a new treatment for HI/HA in children. Dr. Stanley’s collaborator, Dr. Thomas Smith, a biochemist at the Donald Danforth Plant Science Center in St. Louis, MO, performed X-ray crystallography studies of the compound in green tea.

Dr. Stanley and Dr. Changhong Li at CHOP tested that compound, called a polyphenol, in mice and in insulin-producing cells. The study, which appeared Aug. 3 in the Journal of Biological Chemistry, showed that the compound controlled blood sugar levels in the animals.

Future research to explore how green tea compound may be modified to derive a drug for humans

“Our research doesn’t mean that people should automatically expect green tea to treat the rare disease HI/HA,” said Dr. Stanley. “However, with further research, we may be able to modify this green tea compound to derive a drug that can be tested in children with HI/HA. It’s possible that such a derivative may control blood sugar levels in people, as successfully as in mice, with fewer side effects than existing drugs. An even more exciting prospect is that this may lead to a drug that is more effective than existing drugs in treating neurological problems in the disease.”